[Congenital absence of the patella: two cases reported]. / Absence congenitale de rotules: à propos de deux cas.

OBJECTIVES: To remind a rare disease, To emphasize the importance of patellar agenesis because it's a calling sign, To insist on the necessity to know child bony growth. MATERIALS AND METHODS: We reported observations of two negro-african children's, one female and one male, who were both 7-years-old. They were referred for troubles of the walk, knee pains for the male, and for the female frequent falls and hypotonia. We have noted previous history of second degree familial consanguinity and paternal grandfather's ungueal anomalies for the female. RESULTS: For the female, examination showed ligamentary hyperlaxity, anterior flattening and lateral dimple of the knee, so varied skeletal defects. Skeletal radiographies displayed absence of the patella, "console like" aspect of the superior tibial metaphysis. At the male, examination is normal. Standard radiographies showed semilunar opacities and tomodensitometry showed presence of a tissular formation who looks like the patella. Aplasia of the patellar apparatus is evocated for the female, even though for the male, the diagnosis of hypoplasia seems more probable. CONCLUSION: Absence of patella is rare. It can be transient and patellar nucleus appears lately. It can be permanent and in this case, it is isolated whether associated with other osseous malformations. Interest of such observations is in its rarity and its functional impact related to the biomechanical importance of the patella.

[Functional prognosis of stroke in countries in the process of development: Senegal]. / Pronostic fonctionnel des accidents vasculaires cérébraux dans les pays en voie de développement: Sénégal.

OBJECTIVE: To evaluate the functional outcome in patients followed in the department of neurology of Dakar for stroke. MATERIALS AND METHOD: Prospective longitudinal observational study from August 2003 to May 2005 included inpatients or outpatients with stroke one month or less from entry confirmed by computed tomography scan. Patients were followed monthly for 12 months. At each visit, data on functional autonomy measured by the Barthel index and the treatments received were collected. RESULTS: One hundred and seventy patients aged 25 to 90 years (mean 61+/-13 years) were evaluated. The sex ratio was 0.68. A total of 64.7% of strokes involved ischemic cerebral vascular accidents and 35.3% hemorrhagic vascular accidents. Right hemiplegia was present in 55.9%, left hemiplegia in 42.9% and bilateral paresis in 1.2% of patients. A total of 28.8% of patients died between d0 and d30, 50.6% within one year. At entry, the Barthel score was greater than 60 in only 4.7% of patients. After one-year follow-up, 58.3% of the patients had a Barthel score greater than 60, 19.1% had a score between 20 and 40 and 22.6% a score between 40 and 60. Only 53.5% received rehabilitation care. The parameters significantly associated with a functional recovery were age less than 55 years (P<0.05), hemorrhagic vascular accident as opposed to ischemic vascular accident (P<0.05), and earlier rehabilitation care under qualified personnel (P<0.01). DISCUSSION: Our study shows an important rate of mortality during the first year following stroke and the rather young age of our population. The parameters associated with a better functional recovery are comparable to most of the data in the literature. The results of our study suggest that it is possible to improve functional prognosis after stroke by setting measures of prevention of the risk factors and rehabilitation in the early management of hemiplegia.

[Cranial and cervical vertebral mycetoma. Report of three cases]. / Le mycétome crânio-cervical. A propos de trois cas.

Mycetomas inflammatory-like tumors presenting as fistulas found in the skin and soft tissues. Inoculation, generally in tropical areas, occurs by skin injury. The foot is the main infection site. Other uncommon infection sites include the cranial and cervical areas which are rare. We present three cases of cranial and cervical mycetoma. Clinical sign were dominated by headache, cervical pain and cervico-occipital tumefaction with formation of pus and granules (red in two cases, black in one). A motor deficit was noted in one patient. Radiographic examinations including CT scan showed extensive mass lesions, associated with bone destruction. Laboratory tests identified Leptospheria senegalensis in one patient and Actinomadura pelletieri in two. A medicosurgical procedure was performed. The course was unfavourable in one patient. Several factors are important for treating such infections: early diagnosis, improvement of the social and economic environment, use of new drugs.

[Evaluation of the cost of stroke management in Dakar, Senegal]. / Evaluation du coût de prise en charge des accidents vasculaires cérébraux à Dakar--Senegal.

The purpose of this one-year cross-sectional study conducted in 1997 was to estimate the direct cost of stroke management in the Neurology Department of Fann University Hospital in Dakar, Senegal. Data were collected about the type of care services required and related spending. Cost analysis was made in CFA Francs (CFAF), the Senegalese currency (1 USD = 500 CFAF in 1997). A total of 1260 patients were hospitalized in the department including 383 for stroke (30.4%). Mean age was 60.8 +/- 14.2 years and men accounted for 49.2% of the population. The mortality rate was 46.2% among hospitalized patients. A total of 33,573 medical acts were carried out including 12,052 (35.9%) for stroke management. The direct cost of stroke management was 32,614,442 CFAF with a mean cost of 78,426 CFAF per patient. The cost was 18,839 CFAF in the patient care unit (57.8%) and 4,954,635 CFAF in the neuroradiology unit (15.2%). The cost of health care personnel was 19,373,172 CFAF (59.4%) and the cost for drugs and other medical products was 8,253,246 CFAF. Health education programs aimed at increasing awareness of risk factors are needed to reduce the cost of stroke management in this difficult economic period.

[Epidemiological and clinical aspects of amyotrophic lateral sclerosis in neurological clinic of Dakar]. / Aspects cliniques et epidémiologiques de la sclérose latérale amyotrophique a la clinique neurologique de Dakar.

In Africa, little data has been published in Amyotrophic lateral sclerosis (ALS). We conducted a retrospective survey among the in-patients from December 1993 to March 2000 in the Department of Neurology of the University Hospital. This study reported epidemiological and clinical data of ALS. ALS has been diagnosed by El Escorial criteria. 33 patients were 16 to 77 years old. Parental consanguinity was found in 15.2%. On the basis of diagnosis criteria, ALS in our patients were established in 57.6% of cases, probable in 30.3% of cases, possible in 9.12% and suspected in 3% of cases. Evolution after hospitalisation is unknown for the majority of patients. Only one patient died after 12 months of evolution.

[Cerebral palsy in Dakar]. / Infirmité motrice cérébrale à Dakar.

The objective of the study was to describe risks factors of cerebral palsy and to determine clinical forms. From November 1998 to July 2000,we conducted a prospective study to select outpatients showing cerebral palsy symptoms: so 93 children with age between 5 months and 11 years old entered on this study. For 44.08% cerebral palsy was related on pregnancy abnormalities, 32.26% have central nervous system infection and 8.6% have convulsions unknown cause . The cause of cerebral palsy was untraceable in 13.98% of the cases. 82.8% of the children have cerebral palsy associated with other handicaps. The commonest type of cerebral palsy was spastic forms (74.2%) followed by hypotonic cerebral palsy (16.13%), dystoniaathetosis and ataxic forms in 5.37% and 4.3% of the cases respectively. The overall prevalence of epilepsy was 46.23%. The other handicaps were neurosensoriel abnormalities (visual impairment, deafness), speech disorders and behavioural problems. Computerised tomography (CT), performed in 45 cases showed cortical/subcortical atrophy as the commonest brain lesions. The electroencephalographic abnormalities were identified frequently.

[Charcot-Marie-Tooth disease associated with a pyramidal syndrome: clinical, electrophysiological, and neuropathological study of neuro-muscular biopsies in 14 cases]. / La maladie de Charcot-Marie et tooth associée a un syndrome pyramidal: etude clinique, électrophysiologique et neuropathologique des biopsies neuro-musculaires dans 14 cas.

A rare clinical form of Charcot-Marie-Tooth (CMT) disease, estimated at less than 5% by a good many authors, it remains unknown at the neuropathological and genetic level. The prevalance of this form in our areas estimated at 16% allows to discuss its clinical, electrophysiological and neuropathological particularities. To define clinical electrophysiological and neuropathological criteria of neuromuscular biopsies of CMT associated with a pyramidal syndrome. forteen patients presenting a CMT disease associated with a pyramidal sign, sometimes sensory troubles pes cavus and ear and at times eye trouble, an electrophysiological exploration allowed to measure the motor conduction velocity (MCV) of the median nerve and the external popliteal sciatic. A neuromuscular biopsy on the peroneal nerve was performed in all patients with their free consent or that of their parents or relatives when necessary. Semi-thin cuttings coloured with toluidine hue were examined under photonic microscope with x10, x40, and x100 enlargements and the study of the dissociated nervous fibres (teasing) was curried out. The 14 patients aged 8 to 37 years (average 23+/-0.5). 7 females and 7 males clinically presented a CMT disease with a pyramidal syndrome Sensory troubles existed in 8 cases, a lesion of the cranial nerve was observed in 8 cases (II and VII) parental consanguinity existed also in 9 cases. The MCV of the median nerve were more > 40 m/sec in 8 cases whereas in 2 cases they were < 40) m/sec. All muscles showed an atrophy of neurogenous type. In 4 cases the nerves were histologically normal on semi-thin cuttings and <>. There were axonal degeneration lesions in 2 cases only and demyelinating lesions in 6 cases. No onion bulb lesion as observed nor images of regeneration (cluster). The CMT disease associated with a pyramidal syndrome is a clinical entity which constitutes a heterogenous group at the clinical, electrophysiological and anatomo-pathological and probably genetic level. A dominant autosomal transmission and some recessive forms were isolated but the chromosomic anomaly involved remains to be defined.

[Friedreich's disease in the department of neurology in Dakar]. / La maladie de Friedreich dans le service de neurologie de Dakar.

A retrospective study of Friedreich's ataxia was conducted from january 1960 to december 1997. Eighten cases had been collected. Friedreich's ataxia was the second disorder after Pierre Marie's ataxia among inherited ataxia. Parental consanguinity has been found in 27.77% of cases. The sex-ratio was 2.6 for males. 66,67% of Friedreich's ataxia cases began after 25 years. Clinical signs were: cerebellar ataxia, sensitive disorders in 70%, pyramidal syndrom and cardiomyopathy in 22.22%, bones dysmorphy in 50% of cases. Electromyography indicated severe axonopathy of members with decreased somesthesic potentials in six cases. Glycaemia was normal in all cases. Clinical future presented wide variation between one and fourth years old.

[Epidemiological, clinical and progressive aspects of neurological manifestations associated with retroviral infections: eleven year retrospective study]. / Aspects epidémiologiques--cliniques et evolutifs des manifestations neurologiques associées a l'infection rétrovirale: etude rétrospective sur 11 ans.

Through a cohort of 93 neuroaids which has been diagnosed at Dakar in our Neurology Department, the authors evaluated the hospital prevalence of retrovirus, detected socio-demographic factors, related AIDS outline the mean neurological picture and try to correlate survival and neurological involvement of these patients. Among 1151 patients who got retroviral blood test, 93 were seropositive (8.1%). On these repartitions 36 were females (38.7%) and 57 males (61.3%). The age goes from 19 to 76 years old. 45 patients (48.4%) have been found positive for HIV-1 blood test, 21 patients (22.6%) for HIV-2 blood test, 11 patients (11.8%) for both HIV2, 11 patients (11.8%) for HTLV1, 3 patients (3.2%) for both HIV-1 and HTLV1, and 2 patients (2.2%) for both HIV-2 and HTLV1. In our study the transmission of AIDS occur mainly through heterosexual inter course and multiple parternship is a high risk group. The central nervous system deseases represented 68.8% of cases. The pathology were dominated by stroke, myelopathies, meningoencephalotis and spinal cord compression. The peripheral nervous system desease were found in 7.5% of cases. The peripheral facial paralysis occupied the first place in HIV infections of peripheral nervous system deseases (57.1% of cases). When neurological involvement set up the letality is higher for HIV-1 (57% of global letality) and for central system nervous involvement (76.2%).

[Aetiological aspects of neurological diseases in Dakar: follow-up after 10 years (1986-1995)]. / Aspects etiologiques des affections neurologiques à Dakar: bilan de 10 années (1986-1995).

Because of the apparition of new risk factors and numerous progresses in investigation methods, authors take stock of neurological diseases of patients admitted in Clinique Neurologique of Fann during the last ten years. The 8539 cases repartition is as followed: 4736 males and 3803 female. Their ages ranged between 3 and 80 years with 2130 deaths corresponding to 24.94%. Annual mean of admission is 853 +/- 42 cases. Aetiological data are: stroke (3910 cases 45.78% with 60.56% of death). The following is peripheral neuropathieswith 714 cases(8.61%) and the spinal cord compression with 692 cases (8.10%). Comparision of frequencies of different aetiologies with those of previous studies shows that the small capacity of the neurological department is a restricting factor for the entry frequencies of neurological patients; the principal pathologies got small variations from one decade to another. In the opposite, some aetiologies have disappeared (neurological syphilitis, trypanosomiasis, neurocyticercosis, cerebral mycosis). Some eatiologies remain unprecised (infectious diseases, peripheral neuropathies, degenerative diseases).

[Guillain-Barre syndrome in children: experience in the neurology service of Dakar]. / Syndrome de Guillain-Barré chez l'enfant: expérience du service de Neurologie de Dakar.

A retrospective survey has been conducted from january 1979 to january 1999 about the young inpatients file records of the department of neurology of Dakar. 19 cases of Guillain-Barré's syndrome have been collected, representing 2.3% of the children admitted in the department of neurology. The children were 18 months to 16 years old. The sex ratio was 1.4. Infectious disease has been reported in 68.4% of the cases during the 4 weeks preceding the neurological features. The notion of vaccination was found in 5.3% of the cases. The motor deficit was frequent, represented by paraparesis in 63.2% and quadriparesis in 36.8% of the cases. The mean duration of the hospitalisation was 28 days. 52.6% of the patients have completely recovered from their motor deficit and this recovery was unachieved in 36.8%. No fatal event has been detected. The therapeutical means were represented by vitaminotherapy B1B6B12 associated with kinesitherapy. Our results reveal a less severe evolution and better prognosis of Guillain-Barré's syndrome affecting children, instead of the absence of more appropriate means of treatment such as plasma exchange and intravenous immune globulin.

[Neuropediatrics: epidemiological features and etiologies at the Dakar neurology service]. / Neuropédiatrie: aspects épidémiologiques et étiologiques dans le service de Neurologie de Dakar.

Child neurology is a relatively young speciality of neurosciences which is at the frontier of Neurology and Paediatrics. Its development has been impulsed by the diagnosis techniques such as Neurobiology, Genetics, Neuroimaging and pedo-psychology. We conducted a retrospective survey among the in-patients from January 1980 to December 1997 in the service of Neurology of the University Hospital. Have been included children ranged from 0 to 15 years old without any racial, sexual or origin distinctive. In Neurology Department, children of 0 to 15 years old represent 10.06% of the in-patients received from 1980 to 1997. The mortality rate was 9.23%. The diseases are dominated by epilepsy and infantile encephalopathies with 31.02%, infectious diseases with 19.36% represented by tuberculosis, other bacterial, viral and parasitical etiologies, tumors with 10.36%, vascular pathology and degenerative disorders.

[Dorsal cord compression by aspergilloma. Apropos of a case]. / Compression médullaire dorsale par aspergillome. A propos d'un cas.

Spinal cord compressions are mainly due to Pott disease. We present a rare case. A 31 years old woman presenting a spinal cord compression due to an aspergilloma. Our patient presented also a pulmonary aspergillosis. The spinal compression was due to a contiguous extension of aspergillus to the epidural site. The treatment associated surgery and medical treatment (Ketoconazole), with efficient results.